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February 24, 2024
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Study reveals hereditary angioedema experiences among underrepresented populations

Fact checked byKristen Dowd
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Key takeaways:

  • Patients reported Black (33.1%), Hispanic (30.2%) or more than one (18.7%) race or ethnicity.
  • 46.6% and 39.8% reported excellent or good control, with a median of six attacks in the past year.

WASHINGTON — Patients from underrepresented groups experienced improved control of their hereditary angioedema after diagnosis, according to a presentation at the American Academy of Allergy, Asthma & Immunology Annual meeting.

However, these patients often continued to experience challenges in managing their disease, Salomé Juethner, MSN, senior medical director, head of rare genetics, Takeda Pharmaceuticals USA Inc., told Healio.

swollen tongue
Noting a lack of representation in previous studies, researchers surveyed patients from diverse racial and ethnic backgrounds about their experiences with hereditary angioedema. Image: Adobe Stock

“I’ve been working in a lot of underserved types of disease areas,” Juethner told Healio.

Noting that white patients are overrepresented in clinical trials that study many diseases including hereditary angioedema (HAE), Juethner said that data on self-reported outcomes among patients from underrepresented racial and ethnic groups are limited.

“Trials just don’t tend to have as many underrepresented racial or ethnic groups as we would like,” she said.

The researchers then set out to describe and understand how patients with HAE from these groups utilize health care resources, as well as the attack severity, treatment, symptom control and impact on daily activities that they experience.

The need for inclusion

Patient diversity has not been “top of mind” in previous clinical trials investigating HAE, Juethner said, but “there’s a tremendous amount of work that’s going on now to ensure better real representation.”

For example, there are more efforts to consider where clinical trials are being conducted and who may be in those patient pools, she said, bringing them to locations where more diverse populations may be found.

“You want to make sure that your drug is going to work on all patient types,” Juethner said.

Also, she said that it is important to give patients opportunities to participate in trials and get access to new therapies.

“And from a patient-reported outcomes perspective, it’s important to understand every patient’s journey, not just one type of patient,” she said. “Most trials do have quite a bit of patient-reported outcome data that’s collected.”

These considerations led Juethner and her colleagues to this study, she said.

“We realized that it was possible that we did not have a full sense of the HAE patient journey, because what we know so far is very much represented by white patients,” Juethner said.

Juethner and her colleagues reached out to patient advocacy groups and leveraged Takeda’s One Path patient service program to find participants.

Patient characteristics

The noninterventional, observational and web-based survey of adults with a self-reported diagnosis of HAE who identified as members of an underrepresented racial or ethnic group included 139 respondents (mean age, 43.2 years) who met study inclusion criteria, with 133 completing the survey.

“We hoped for 50 patients, and we ended up with 139 very quickly, which really pointed to us that patients wanted to share their story and tell us about their experience,” Juethner said.

With 82% of the 133 respondents who reported gender identifying as women, race and ethnicity proportions in this group included 33.1% African American or Black; 30.2% Hispanic, Latin American, Latine or Latinx; and 18.7% selecting more than one race or ethnicity.

Proportions also included 5% each for Alaska Native, American Indian or Native American; Asian, Asian American, East Asian or South Asian; and race or ethnicity not listed. Middle Eastern or North African (1.4%) and multiracial or multiethnic (1.4%) also were reported.

Mean age at diagnosis of HAE was 23.3 years (standard deviation [SD], 14.1) for 133 patients in the cohort, and 95% said they were diagnosed after experiencing their first HAE attack or symptoms.

Numbers of HAE attacks in the previous year (n = 135) included zero to five for 47.4%, six to 20 for 26.7% and more than 20 for 25.9%.

Family histories of HAE (n = 133) included parents or grandparents for 46.6%, siblings for 30.1%, children for 25.6% and other for 17.3%, with 24.1% reporting no family history and 6% saying they were not sure.

Annual household incomes before taxes (n = 133) included less than $15,000 (12.8%), $15,000 to $24,999 (11.3%), $25,000 to $49,999 (22.6%), $50,000 to $99,999 (23.3%), $100,000 to $149,000 (10.5%) and $150,000 or greater (6%).

Reported health insurance coverage (n = 133) included private or commercial health insurance (66.9%), Medicaid (21.1%), Medicare or Medi-Gap (15.8%), other coverage (3.8%), military health care (1.5%) and no coverage of any type (1.5%).

HAE management

The survey also found that 101 of 135 (74.8%) individuals in the cohort took medication for long-term prophylaxis (LTP) on an ongoing basis during the previous year, with 80% taking it all the time, 15% taking it most of the time and 5% taking it some of the time.

Patients reported taking a mean of 2.6 (SD, 3.6) different on-demand or rescue medications and a mean of 2.6 (SD, 2.6) LTP medications since their HAE diagnosis as well.

Although 62 of 133 (46.6%) patients said they had excellent symptom control and 53 of 133 (39.8%) said they had good control, the researchers said there was considerable variability in the number of attacks over the previous year, with a median of six (interquartile range [IQR], 2-22).

Once patients had the diagnosis, and had been connected with an HAE expert, they felt they had good or excellent control of their HAE, and that was really key,” Juethner said. “But even though they felt like they had good and excellent control, there were still people who were having attacks.”

By percentage, 10.4% reported no attacks; 19.3% reported one or two; 17.8% reported three to five; 12.6% reported six to 10; 14.1% reported 11 to 20; 16.3% reported 21 to 50; and 9.6% reported more than 50 attacks.

By severity, patients reported medians of three (IQR, 1-10) mild attacks, two (IQR, 0-6) moderate attacks and zero (IQR, 0-3) severe attacks. Also, 63 of 120 patients (52.5%) of those who had one or more attacks treated every attack with medication.

Common reasons for not treating any attacks in the previous year included attacks that were not severe enough (82.5%), worries about using up medication (40.4%), running out of medication (29.8%) and not wanting to get an injection or infusion (26.3%).

Other reasons included concerns about costs of treatment or medication (15.8%), not having their medication with them (14%), inability to get to their medication (10.5%), not thinking the medication would work (5.3%) and other (8.8%).

The survey also revealed a mean of 6.8 (SD, 10.9) visits to doctors or health care providers before a diagnosis among the patients who were not diagnosed until after their symptoms had begun.

Similarly, these patients reported mean annualized rates of 5.3 (SD, 10.6) ED or urgent care visits and 2.7 (SD, 7.7) overnight hospital admissions before they were diagnosed.

Once patients with one or more attacks in the previous year had a diagnosis and disease management began, these means fell to 2.6 (SD, 8.4) for ER and urgent care visits and 0.4 (SD, 1.5) for overnight hospital admissions.

When patients (n = 135) were asked to rate the impact that their HAE had on their ability to perform regular daily activities over the previous year on a scale of zero (no impact) to 10 (complete prevention of daily activities), the mean rating was 3.7 (SD, 2.8).

By scale, 11.1% said there was no impact with a rating of zero, 18.5% rated the impact at one on the scale, 14.1% rated the impact at five, 0.7% rated it at nine, and 5.9% rated it at 10, with complete prevention of their daily activities.

Conclusions, next steps

These findings indicate that patients with HAE from underrepresented groups experience improved control after diagnosis and treatment, with less health care resource utilization among those who had one or more attacks in the previous year, the researchers said.

Yet this appearance of a difference between patient perceptions that their disease is well controlled and the persistence of attacks indicate that minimal improvements in attack rates may lead to improvements in how they perceive their health.

“There was that little bit of a disparity between, ‘I have good or excellent control, but I’m still having attacks,’” Juethner said. “We’d like to understand that a little more. I think that’s a good next step.”

Reducing the time from symptom onset to diagnosis is another goal, Juethner continued.

“That is really the top priority,” she said. “Let’s shorten that time to diagnosis and make sure that there are experts available to you.”

Cultural competency can play a key role in these improvements, Juethner said, as physicians work to meet patients’ needs when they meet with them. For example, she said, educational materials written in their language and diversity in medical personnel would have an impact.

“Patients like to see someone who looks like them,” she said.

Physicians should be mindful of social determinants of health in treating patients from underrepresented backgrounds as well, Juethner said, adding that she and her colleagues will be investigating these areas too.

“It’s not just race that affects your health outcomes,” she said. “That is our next step. That’s what we’re currently collecting data on.”

The results of these additional studies may inform later policies, Juethner said.

“What action should be taken from an education perspective? Maybe it’s certain CMEs targeted to certain groups. Educational materials for patients,” she said.

These educational efforts among patients and physicians alike would share the goal of shortening time to diagnosis, Juethner said, as Takeda has supported continuing education about HAE for primary care providers and ED doctors.

“We actually worked with a group of gastroenterologists who paired up with our HAE experts, and we wrote a manuscript on the misdiagnosis of HAE in gastroenterology because they have so many abdominal symptoms,” she said. “So, we are definitely aiming out beyond our allergy and immunology experts to try to find those patients earlier.”

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